ArticlePulmonary circulation2017
Chronic use of PAH-specific therapy in World Health Organization Group III Pulmonary Hypertension: a systematic review and meta-analysis.
Article in Pulmonary circulation, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 43 papers.
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Who cites it
43 citing papers in PubMed, 77 citations in OpenAlex.
- Inhaled treprostinil in pulmonary hypertension associated with COPD: PERFECT study results.The European respiratory journal · 2024Trial
- Right Ventricular Phenotyping Can Lead to Pulmonary Vascular Therapy Response in Those with Pulmonary Hypertension with COPD: A Single-Center Cohort Study.Journal of cardiovascular development and disease · 2025Article
- Molecular Pathogenesis of Connective Tissue Disease-Associated Pulmonary Arterial Hypertension: A Narrative Review.Biomolecules · 2025Review
- Significance of Normal Lung Volume on Quantitative CT Imaging Analysis in Group 1 and Group 3 Pulmonary Hypertension.CHEST pulmonary · 2024Article
- Pulmonary hypertension associated with lung diseases.The European respiratory journal · 2024Review
- COPD associated pulmonary hypertension: A post hoc analysis of the PERFECT study.Pulmonary circulation · 2024Article
- The efficacy and safety of herbal formulas for adults with pulmonary hypertension combined with chronic obstructive pulmonary disease: a systematic review and meta-analysis involving 1,865 participants.Journal of thoracic disease · 2024Article
- Urolithin A Protects against Hypoxia-Induced Pulmonary Hypertension by Inhibiting Pulmonary Arterial Smooth Muscle Cell Pyroptosis via AMPK/NF-κB/NLRP3 Signaling.International journal of molecular sciences · 2024Article
- Cluster analysis identifies novel real-world lung disease-pulmonary hypertension subphenotypes: implications for treatment response.ERJ open research · 2024Article
- Pulmonary Vasodilator Therapy Is Associated with Decreased Mortality in Patients with Chronic Lung Disease and Severe Pulmonary Hypertension.Journal of cardiovascular development and disease · 2024Article
- Functional Roles of CD26/DPP4 in Bleomycin-Induced Pulmonary Hypertension Associated with Interstitial Lung Disease.International journal of molecular sciences · 2024Article
- Pulmonary hypertension in the setting of interstitial lung disease: Approach to management and treatment. A consensus statement from the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative-Group 3 Pulmonary Hypertension.Pulmonary circulation · 2024Review
- Proteomic biomarkers for survival in systemic sclerosis-associated pulmonary hypertension.Respiratory research · 2023Article
- Pulmonary Hypertension in Interstitial Lung Disease: Updates in Disease, Diagnosis, and Therapeutics.Cells · 2023Review
- Multidisciplinary team discussion: the emerging gold standard for management of cardiopulmonary complications of connective tissue disease.Internal medicine journal · 2023Review
- Pulmonary Hypertension: A Contemporary Review.American journal of respiratory and critical care medicine · 2023Review
- Impact of targeted pulmonary arterial hypertension therapies in severe pulmonary hypertension in chronic lung diseases.ERJ open research · 2023Article
- Mechanisms of hypoxaemia in severe pulmonary hypertension associated with COPD.The European respiratory journal · 2023Article
- Inflammation and immunity in the pathogenesis of hypoxic pulmonary hypertension.Frontiers in immunology · 2023Review
- Non-invasive detection of severe PH in lung disease using magnetic resonance imaging.Frontiers in cardiovascular medicine · 2023Article
Corrections and comments
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Authors and funding
5 authors at 1 institution in 1 country.
Funding
Abstract
Pulmonary hypertension (PH) complicating chronic obstructive pulmonary disease (COPD-PH) and interstitial lung disease (ILD-PH) (World Health Organization [WHO] Group III PH) increases medical costs and reduces survival. Despite limited data, many clinicians are using pulmonary arterial hypertension (PAH)-specific therapy to treat WHO Group III PH patients. To further investigate the utility of PAH-specific therapy in WHO Group III PH, we performed a systematic review and meta-analysis. Relevant studies from January 2000 through May 2016 were identified in the MEDLINE, EMBASE, and COCHRANE electronic databases and www.clinicaltrials.gov. Change in six-minute walk distance (6MWD) was estimated using random effects meta-analysis techniques. Five randomized controlled trials (RCTs) in COPD-PH (128 placebo or standard treatment and 129 PAH-medication treated patients), two RCTs in ILD-PH (23 placebo and 46 treated patients), and four single-arm clinical trials (50 patients) in ILD-PH were identified. Treatment in both COPD-PH and ILD-PH did not worsen hypoxemia. Symptomatic burden was not consistently reduced but there were trends for reduced pulmonary artery pressures and pulmonary vascular resistance with PAH-specific therapy. As compared to placebo, 6MWD was not significantly improved with PAH-specific therapy in the five COPD-PH RCTs (42.7 m; 95% confidence interval [CI], -1.0 - 86.3). In the four single-arm studies in ILD-PH patients, there was a significant improvement in 6MWD after PAH-specific treatment (46.2 m; 95% CI, 27.9-64.4), but in the two ILD-PH RCTs there was not an improvement (21.6 m; 95% CI, -17.8 - 61.0) in exercise capacity when compared to placebo. Due to the small numbers of patients evaluated and inconsistent beneficial effects, the utility of PAH-specific therapy in WHO Group III PH remains unproven. A future clinical trial that is appropriately powered is needed to definitively determine the efficacy of this widely implemented treatment approach.
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